19 research outputs found
Serous Cystadenocarcinoma Arising in Presumed Vitelline Duct Remnant: A Case Report and Implications in the Management of Cancer of Unknown Primary
No full textBackground. Malignant neoplasms arising in Meckel’s diverticulum, a vitelline duct remnant, are rare yet well-documented. Case Presentation. A 53-year-old previously healthy female presented with an enlarging midline abdominal wall mass. A computed tomography scan revealed a mass involving the linea alba, bilateral rectus abdominis, and subcutaneous fat. Extensive clinical workup failed to demonstrate other lesions, except local and paratracheal/hilar lymphadenopathy. Histopathologic examination of the resected tumor demonstrated a spectrum of serous neoplasia including serous cystadenoma, papillary serous carcinoma with numerous Psammoma bodies, and a poorly differentiated component. Immunophenotypically, the tumor cells were strongly positive for CK7, CK19, CA19.9, and MUC1 but negative for other lineage markers, findings suggestive of pancreatobiliary type differentiation. The patient died of the disease one year after the initial presentation despite chemotherapy, radiation, and surgery. Conclusion. We present a case of adenocarcinoma arising from the anterior midline abdominal wall, from presumed vitelline duct remnant, with histologic and immunophenotypic features of serous cystadenocarcinoma of pancreatobiliary origin. Though the origin from vitelline duct remnant is difficult to prove in this single case, understanding tumorigenesis of embryonic remnant origin is potentially important to improve the management of cancer of unknown primary
Immunohistochemical Expression of Embryonic Stem Cell Markers in Malignant Rhabdoid Tumors
No full text Malignant rhabdoid tumor is a highly aggressive pediatric neoplasm molecularly characterized by inactivating mutations of the SMARCB1 gene, a potent tumor suppressor and member of the SWI/SNF chromatin remodeling complex. It has been suggested that oncogenesis in SMARCB1-deficient cancers, such as malignant rhabdoid tumors, is driven not by the loss of SWI/SNF function but by an aberrant functioning of the BRG1-containing SWI/SNF complex. Since Brg1 is required for self-renewal and pluripotency of mouse embryonic stem cells, we hypothesized that the human malignant rhabdoid tumors may express pluripotency genes such as SALL4, LIN28, OCT3 and OCT4 (OCT3/4), NANOG, and TCL1. To test this hypothesis, we studied the immunohistochemical expression of SALL4, LIN28, OCT3/4, NANOG, and TCL1 in 11 malignant rhabdoid tumors of the central nervous system (atypical teratoid/rhabdoid tumors) and 5 malignant rhabdoid tumors of the kidney. Of the 16 malignant rhabdoid tumors, 14 (88%) tumors showed robust SALL4 and/or LIN28 expression. No tumor showed any significant OCT3/4, NANOG, or TCL1 expression. Our results suggest that malignant rhabdoid tumors may arise from and/or share features with embryonic stem cells or germ cells. </jats:p
Intracranial Solitary Fibrous Tumor with Pseudopapillary Architecture: An Uncommon Tumor with Unusual Histopathology
No full textSolitary fibrous tumor (SFT) is an uncommon soft tissue neoplasm first described in the pleura in 1931 and later recognized in other anatomical locations. Involvement of the central nervous system is rare; there are approximately 250 cases of central nervous system SFTs in the English literature. To the best of our knowledge, this is the first report of an intracranial SFT showing pseudopapillary architecture. The patient was a 73-year-old male who presented with recent onset altered mental status. Imaging studies showed a large left posterior parieto-occipital region intradural extra-axial mass with significant mass effect in the adjacent brain. Neuropathologic examination demonstrated a spindled mesenchymal neoplasm with variable cellularity and prominent collagen deposition. In areas, the tumor was discohesive, imparting a prominent pseudopapillary architecture. Entrapped brain parenchyma was present, indicating brain infiltration. The tumor cells were positive for CD34, CD99, vimentin, BCL-2, and STAT6; EMA was negative. Both the Ki-67 and mitotic indices were low, and anaplastic nuclear features were absent. To the best of our knowledge, this is the first example of an intracranial SFT with pseudopapillary architecture. Occasional extracranial SFTs showing papillary features have been reported. The significance of this architectural pattern is unclear and may be elucidated by future studies. [N A J Med Sci. 2016;9(4):181-186. DOI: 10.7156/najms.2016.0904181] Key Words: solitary fibrous tumor, central nervous system, intracranial, pseudopapillar
72-year-old man with right homonymous hemianopia and gait instability
No full textAn elderly man presented with right homonymous hemianopia and gait instability. He was found to have a left occipital ring enhancing lesion that was resected. Neuropathologic examination demonstrated a Cladophialophora bantiana brain abscess (cerebral phaeohyphomycosis).[Image: see text
Aggressive primary hepatic histiocytic sarcoma: case report and literature review
No full textHistiocytic sarcoma is an uncommon non-Langerhans histiocyte disorder of mature tissue histiocytes. The authors presented an example of this rare tumor in a 14-year-old girl who presented with left upper quadrant pain, loss of appetite, and weight loss. A large 18 cm × 10 cm heterogeneous solid and cystic enhancing mass was found in the left lobe of the liver. Based on the histomorphology and positivity for histiocyte-specific markers in a needle biopsy, a diagnosis of histiocytic sarcoma was made. Chemotherapy was initiated, but the tumor did not respond well, and she died about 7 weeks following initial diagnosis with multi-organ failure. At autopsy, the tumor showed extensive necrosis, with no evidence of metastatic spread. In conclusion, the diagnosis of histiocytic sarcoma is challenging, and requires a high index of suspicion, with an appropriate panel of confirmatory immunohistochemical stains. Recognition of this rare tumor is important because of its poor response to chemotherapy and high mortality
